1 / 14

Epiderrmolysis bullosa pruginosa

Epiderrmolysis bullosa pruginosa. Epidermolysis bullosa pruriginosa. Dystrophic epidermolysis bullosa (DEB) pruriginosa (DEB-Pr) is a rare variant of DEB due to COL7A1 mutations. Both AR and AD inheritance and sporadic inheritance patterns have been recognized.

lhaughton
Download Presentation

Epiderrmolysis bullosa pruginosa

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Epiderrmolysis bullosa pruginosa

  2. Epidermolysis bullosa pruriginosa Dystrophic epidermolysis bullosa (DEB) pruriginosa (DEB-Pr) is a rare variant of DEB due to COL7A1 mutations. Both AR and AD inheritance and sporadic inheritance patterns have been recognized. EB pruriginosa presents either at birth or during infancy or childhood but in some cases appears later.

  3. Clinically There are severe itching and violaceous papular and nodular lesions, often in a linear arrangement , mainly on extremities. Milia, nail dystrophy and intact blisters are rarely seen. The cause of severe itching is unknown. DD Lichen simplex chronicus. Hypertrophic LP. Cutaneous amyloidosis. Dermatitis artefacta.

  4. Treatment is aimed at controlling itching and halting the progression of cutaneous lesions and includes : Potent topical steriods. IL triamcinolone. Topical tacrolimus. Antihistamines. Systemic steriods. Cyclosporin. Thalidomide.

  5. A 25-year-old male patient presented with itchy skin lesions of 5 years duration, with gradual onset and progressive course. The patient gave family history with similar condition.

  6. E/M LL BC LD

More Related